Closing patellar tendon defects after anterior cruciate ligament reconstruction: absence of any benefit

The most common graft in anterior cruciate ligament (ACL) surgery involves using the central one-third of the patellar tendon. Knowledge concerning the postoperative disability after harvesting the patellar tendon is, however, limited. The aim of this study was to evaluate the impact patellar tendon suture and bone grafting of the patellar bone defect might have in terms of functional outcome and patellofemoral pain after harvesting the bone-tendon-bone graft, compared with leaving the harvested site non-sutured and non-grafted. Sixty patients, scheduled for arthroscopically assisted ACL reconstruction, were randomly allocated to two groups. In group I, suture of the patellar tendon and bone grafting of the patellar defect were performed. In group II, the tendon gap and the patellar defect were left open. Preoperatively, there was no significant difference between the groups when comparing objective knee stability, as measured with a KT-1000 laxity meter, Lysholm score, Tegner activity level, IKDC score, or patellofemoral pain score. Both groups had a significantly improved Lysholm score at the 2-year follow-up, without any difference between them. Tegner's activity level was significantly lower at follow-up, compared with the pre-injury level in both groups. The patellofemoral pain score improved significantly after the reconstruction, without any difference between the groups. Ultrasonography did not reveal any difference between the groups in terms of healing of the tendon gap. This study revealed no differences in donor site morbidity, functional outcome, patellofemoral pain score or knee joint stability between the two treatment groups. The conclusion is that suture of the patellar tendon and bone grafting of the patellar defect do not improve the functional results or reduce donor site morbidity after arthroscopically assisted ACL. Received: 17 December 1996 Accepted: 30 July 1997     

Use of amniotic grafts in the repair of gastroschisis

This article describes the use of amniotic grafts (AG) in the repair of large abdominal wall defects in newborns with gastroschisis. From 1988 to 1995, 22 newborns with gastroschisis underwent surgical repair. In 12 primary closure (PC) was performed; in 10 the abdominal wall defect was covered with an AG. A double layer of AG was used and the graft was additionally covered with a silastic silo in 8 cases. The overall mortality was 14%; 3 children died from necrotizing enterocolitis or sepsis after AG. However, the difference in mortality of newborns with PC versus AG was not statistically significant. Patient characteristics, the postoperative courses, nd the frequency of complications were similar after PC and AG. It is concluded that AG has no negative impact on the postoperative course and yields a low overall mortality. In our opinion there are several advantages in using the AG technique: it is an autoplastic material that is readily available without costs, reefing and removal is unnecessary, and there is a potentially low rate of adhesions.     

Single 5'green-3'red Hybrid Gene in Protanopia

Purpose: To disclose the structure of visual pigment gene for a protanopia with specific variation.Methods: Exon 5 fragments of the red andgreen visual pigment genes from the protanopia with specific varnation as well as controls were amplified by poly-merase chain reaction (PCR). The PCR products were put through heteroduplex-SSCP analysis and PCR-RFLP (restriction fragement length polymorphism) analysis to clarify the specific variation. The specific variation of the exon 5 DNA fragment from the protanopia was identified by sequencing.Results: A novel 5'green-3'red hybrid gene fragment without the normal red and green visual pigment gene was discovered in the protanopia. He should only have a single visual pigment gene, 5'green-3'red hybrid gene, on his X chromosome. The fusion point is between codon 285 and codon 296 in exon 5. Conclusion : Unequal intragenic recombination may occur in exon 5 as well as its upstream. A 5'green-3'red hybrid gene may present independently on the X chromosome without ac     

Mental retardation,structural anomalies of the central nervous system,anophthalmia and abnormal nares: A new MCA/MR syndrome of unknown cause

We report on 2 unrelated Brazilian girls, born to nonconsanguineous parents, and presenting structural central nervous system defects, hydrocephaly, macrocephaly, craniosynostosis, prominent forehead, anophthalmia, and abnormal nares. These patients may have a previously undescribed recurrent-pattern cerebro-oculo-nasal syndrome. © 1993 Wiley-Liss, Inc.     

CLINICAL OBSERVATION OF 74 REVASCULARIZED OSTEOMYOCUTANEOUS FLAP GRAFTS TO RECONSTRUCT THE MANDIBULAR DEFECTS

Vascularized osteomyocutaneous transplantation using the technique of microvascular anastomosis has shown many advantages. For the purpose of immediate reconstruction of composite defects of the mandible, 74 osteomyocutaneous flaps have been used in the last six years. They included 43 iliac osteomyocutaneous flaps pedicled to the deep circumflex iliac artery, 16 latissimus dorsi osteomyocutaneous flaps pedicled to the thoracodorsal artery and 15 pectoralis major asteomyocutaneous flaps pedicled to the acromiothoracic artery. The survival rate of the former was 90.7% (39/43), and the latter two 71% (22/31). The factors influencing the survivalrate of these osteomyocutaneous flaps have been analyzed; the complications of the recipient and donor sites have been presented; the long term results have been discussed. The authors conclude: mandibular reconstruction by using osteomyocutaneous flap grafts has the advantages of resistance to infection, fast bone healing and survival in a poorly vascularized recipient bed. The iliac osteomyocutaneous flap is better than the other two in survival ability.     

Charcot-Marie-Tooth phenotype produced by a duplicated PMP22 gene as part of a 17P trisomy-translocation to the X chromosome

The Charcot-Mane-Tooth disease type 1A (CMTlA) phenotype is most often associated with a 1.5 megabase (mb), tandem duplication of chromosome 17 band p12 (17˜12). The prevailing hypothesis is that the demyelinating neuropathy results from a dosage effect of the peripheral myelin protein gene PMP22 which is included within this duplication. We present a patient with clinical and electrophysiological features ofCMTlA in whom an extra PMP22 gene resulted from a rare unbalanced translocation of 17p to the X chromosome. This finding further supports the hypothesis of gene dosage as the basis for CMTlA. More-over, this case highlights the importance of fluorescence in siiu hybridization (FISH) as an alternative molecular technique in the diagnosis of CMTlA.     

Fine Structure of A: Autonomic Nerve Fibers and Terminals in Human Myocardium; and B: Myocardial Changes in Congenital Heart Disease

In a histological and fine structural study of right atrial biopsy specimens from 31 patients with rheumatic heart disease (RHD), aged 7 to 46 years, and 11 patients with congenital heart disease (CHD), aged 3 to 36 years, nerve fibers or endings were seen by electron microscopy in 11 specimens. There was concurrence of ordinary axons along with terminals bearing pale cholinergic or dark adrenergic synaptic vesicles. Smaller and denser cholinergic vesicles suggested proliferation followed by exhaustion of such nerve endings. The closest proximity of nerve terminal to muscle fiber was about 100 nm. In one RHD specimen a “specific terminal cell” was present between a nerve ending and muscle fiber; in another a possible neuromuscular contact was developing at the surface of a regenerating small muscle fiber with a few myofilaments. Unmyelinated axons amidst increased subendocardial and subepicardial collagen, with prominent fibroblasts and depleted muscle fibers, were seen more frequently in specimens of CHD. Loss of myofibrils and accumulation of mitochondria, with infrequent formation of lipofuscin bodies, characterized degenerating muscle fibers in CHD also, although to a lesser degree than in RHD (reported earlier, 1985). The myocardial blood vessels in CHD tended to have pale swollen endothelial cells and narrowed lumen. The most severely affected cases of CHD were those with (1) a very wide atrial septal defect (ASD), (2) ventricular septal defect (VSD) with vegetations near the defect, (3) 1 infundibular pulmonary stenosis, and (4) Fallot's tetralogy.     

Congenital heart defects in CHARGE: The molecular role of CHD7 and effects on cardiac phenotype and clinical outcomes

CHARGE syndrome is characterized by a pattern of congenital anomalies (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth, Genital abnormalities, and Ear abnormalities). De novo mutations of chromodomain helicase DNA binding protein 7 (CHD7) are the primary cause of CHARGE syndrome. The clinical phenotype is highly variable including a wide spectrum of congenital heart defects. Here, we review the range of congenital heart defects and the molecular effects of CHD7 on cardiovascular development that lead to an over‐representation of atrioventricular septal, conotruncal, and aortic arch defects in CHARGE syndrome. Further, we review the overlap of cardiovascular and noncardiovascular comorbidities present in CHARGE and their impact on the peri‐operative morbidity and mortality in individuals with CHARGE syndrome.     

Autologous rib perichondrial grafts in experimentally induced osteochondral lesions in the sheep-knee joint: Morphological results

Summary The purpose of the present study was to examine the fate of autologous perichondrial grafts after transplantation into cartilage lesions in weight-bearing joints. Osteochondral lesions were made in the articular surface of knee joints in 36 sheep. The defects were filled with autologous rib perichondrial grafts which were secured by either collagen sponges (12 animals) or fibrin glue (12 animals). Defects without perichondrial grafts served as controls (12 animals). Following 1 week of immobilization of the operated leg, the plaster was removed and the animals were allowed to move freely. Animals were sacrificed after 4, 8, 12 and 16 weeks. The grafts were removed and investigated histologically. In contrast to weight-bearing areas and control defects, hyaline-like cartilage formation was seen in non-weightbearing areas after 4 weeks. This newly formed cartilage revealed strong metachromasia following staining with acidic toluidine blue and reacted positively with periodic acid-Schiff, indicating de novo synthesis of proteoglycans and glycoproteins. Scanning electron microscopy and examinations with polarized light confirmed a hyaline cartilage-like architecture for the surface area as well as for the fibre orientation of the whole graft. Enzyme histochemistry for alkaline and acid phosphatase activity showed positive reactivity only at the base of the transplants.     

Occurrence of neural tube defects among first-, second-, and third-degree relatives of probands: Results of a United States study

Data on the occurrence of neural tube defects in first-, second-, and third-degree relatives of probands were collected in a United States study. The proportions of affected individuals were 3.2%, 0.5%, and 0.17% respectively. These findings are compared to those from other recent North American studies, and differences are discussed. It is pointed out that accurate recurrence risk figures may not be available, and that caution should be used when counseling families with relatives who are affected with NTD.